Saturday, June 6, 2009

I have not actually done the wean itself yet. It comes after pituitary surgery!!

I found this helpful in explaining the overall hormone replacement process, and why it is "common" for Cushies to end up in the ER after pituitary surgery. I cannot attest to the tapering schedule and amounts of hydrocortisone to take and when. Please consult with your doctor. ~Melissa

The experience of cortisol withdrawal during the first several months after being cured of Cushing's can be very unpleasant. During this period, cortisol must be given back to the patient and then tapered off over time. Classic symptoms of this withdrawal process include fatigue, aching, and depression. Abdominal pain, nausea and vomiting, and dizziness are indicators that the cortisol withdrawal is occurring too quickly (adrenal insufficiency is occurring) and the hydrocortisone taper should be slowed.

There are two main phases that a cured patient will go through after surgery. The first is the cortisol withdrawal phase. The features of Cushing's disease are due to cortisol excess and the body reacts to having the cortisol withdrawn. To make the process tolerable (and safe), large doses of glucocorticoids are given to the postoperative patient and then tapered over a month or so as symptoms allow. This can be an awful experience for the patient marked by fatigue, depression, and body aches. Headache may be present but if severe (especially if accompanied by nausea and dizziness) may indicate that the taper is happening to quickly. After the initial withdrawal phase, the second phase is marked by the temporary need for continued glucocorticoid replacement (typically 20 mg hydrocortisone or 5 mg prednisone) until the hypothalamic/pituitary/adrenal (HPA) axis awakens. This phase may last many months and may last over a year. Until this axis awakens, the patient is adrenally insufficient and should be treated accordingly.

Assessment of CureJust as the initial diagnosis of Cushing's disease can be difficult to make, "proving" someone has been cured of Cushing's can be very challenging as well. One early indicator of biochemical cure is the measurement of very low (or undetectable) post-operative morning cortisol levels (typically measured 72 hours after surgery). In this post-operative setting, morning cortisol levels are typically low because normal ACTH producing cells in the pituitary have been suppressed by elevated serum cortisol levels. Therefore, the removal of the ACTH secreting tumor leaves no source of ACTH, the adrenal glands are no longer stimulated and cortisol levels plummet. It is important to note that as high as 30% of patients with long term cure of cushing's disease do not have a history of undetectable 72 hour post-operative serum cortisol levels. Therefore, if 72-hour post-operative cortisols are not below 5 ug/dl, further evidence should be obtained to confirm the presence or absence of cure.

After a patient has been tapered off replacement glucocorticoids post-operatively, it is important to demonstrate the normalization of the tests that were initially used to diagnose Cushing's disease. For example if a patient had a significantly elevated 24-hour UFC pre-operatively, it should normalize if a cure was obtained. Other evidence of cure is the dramatic resolution of the features of Cushing's disease. For example, many patients note a dramatic weight reduction in the first few months after surgery. By contrast, even in the context of a biochemical cure, features may resolve slowly. Even in the context of a biochemical cure, the symptoms of fatigue and depression can persist for many months since they are also the symptoms of cortisol withdrawal. It is important to note that even with an incomplete cure (i.e., not all the pituitary tumor was removed), many symptoms will begin to resolve at first but they usually return over time.

In general, when a cure is obtained, most of the features of Cushing's disease will reverse over time. Some of these changes can be dramatic. Some may take years. Of note, the need for medical treatment for high blood pressure and diabetes should be evaluated closely and will likely need to be tapered.

Finally, even in the hands of an excellent neurosurgeon, there is a recurrence rate of about 10% over time for Cushing's disease. Therefore, even in the context of a "biochemical cure", Cushing's patients should be monitored by history, physical, and biochemistry over time for possible recurrence of their disease. Work up and treatment are similar to those done at initial presentation.

Long-term Course and Potential Complications

Cured Cushing's are typically delighted with the resolution of most of their Cushing's symptoms. This can, however, take from months to even years to fully happen. As stated above, recurrence does occur and should be watched for by the patient and their clinician. Associated diseases such as diabetes mellitus, hypertension, and osteoporosis should be followed (and treated if necessary) although each may resolve fully over time as well.

Question: I have been trying to taper my replacement dose of hydrocortisone following pituitary surgery, and I have ended up in the Emergency Room several times due to adrenal insufficiency. I have been told that for my adrenal gland to start producing cortisol and working properly again, I need to be this low. Is that true? What tapering protocol would you recommend and what tests should be performed during the tapering phase?

Answer: These are very good questions. Tapering of hydrocortisone following the removal of an ACTH-producing pituitary tumor, bilateral adrenalectomy or a single Cortisol secreting adrenal tumor results in profound hypothalamic pituitary adrenal suppression, especially when it is successful. The reawakening of this axis may take six to nine months, sometimes longer.

In the interim, patients need to be replaced with glucocorticoid therapy. There is an additional confounding problem and that is when you have Cushing's, your body gets used to higher doses of glucocorticoids; therefore, such high doses need to be continued above replacement doses immediately after surgery and then slowly tapered.

Right after surgery we replace with Hydrocortisone 60 to 80 mg a day for two weeks and start a taper, diminishing 10 to 20 mg every 10 to 14 days, until you are down to physiologic, which is in the neighborhood of 15 to 20 mg per day.

We like to do a Cortrosyn stimulation test every two months to plot the hypothalamic pituitary adrenal recovery. Morning plasma cortisol before taking oral cortisone may also be useful, but we prefer the cortrosyn test. Once the Cortrosyn stimulation test is in the normal range, then Hydrocortisone can be safely discontinued, or tapered to a lower dose, then discontinued.

We do not suggest longer acting steroids, such as Prednisone, which might further prolong pituitary adrenal suppression. The use of Hydrocortisone alone is what is suggested immediately after successful surgery. There is not only a need for Glucocorticoid replacement to avoid adrenal insufficiency, there is a withdrawal from the high endogenous levels. When you withdraw from steroids, even though you are in the normal range for replacement, you will have symptoms of steroid withdrawal which include fatigue, depression, and muscle and joint aching. This is why endocrinologists will tell you that you will feel worse from steroid withdrawal after successful surgery than you felt with the Cushing's syndrome. The steroid withdrawal symptoms can last up to two years and they are again, fatigue, depression, and aching.

There is no good evidence that taking less steroid, or no steroid, accelerates the hypothalamic pituitary adrenal axis recovery. Certainly you cannot use high doses without continuing to suppress the pituitary adrenal axis, but in general, completely stopping steroids is not suggested, since adrenal insufficiency would be predicted under those sets of circumstances and also aggravation of severe withdrawal symptomatology.

In summary, it is important to treat adrenal insufficiency symptoms of lightheadedness and nausea, and also distinguish that from steroid withdrawal symptoms, which are fatigue, depression, and achiness, even though you might be protected from adrenal insufficiency.

I'm sharing a few links that may be helpful. I have collecting these websites for months while preparing for pituitary surgery. There are so many articles out there. Keyword searches are your friend. Mix it up. An abundance of information awaits you. Ex: pituitary surgery, transphenoidal, cortisol hormone replacement, post op pituitary surgery. Just use your imagination, and it will appear! Dedicate yourself to learning as much as you can about your condition. Knowledge is power.

**These links are included for you to get a sense of how different facilities manage their patients after pituitary surgery. The inclusion should not be substituted for medical advice. Please follow your doctors' care.

The pituitary is a small, pea-sized gland located at the base of the brain that functions as "The Master Gland." From its lofty position above the rest of the body it sends signals to the thyroid gland, adrenal glands, ovaries and testes, directing them to produce thyroid hormone, cortisol, estrogen, testosterone, and many more. These hormones have dramatic effects on metabolism, blood pressure, sexuality, reproduction, and other vital body functions. In addition, the pituitary gland produces growth hormone for normal development of height and prolactin for milk production.

The pituitary hangs from the hypothalamus, a part of the brain located immediately above it, by a thread-like stalk that contains both blood vessels and nerves. Hormones produced in the hypothalamus carry signals or messages to the pituitary gland. The hypothalamus serves as a "switching station" to relay signals from many different parts of the brain to the pituitary gland, which in turn transmits its own signals to various parts of the body. The pituitary is divided into a larger anterior region (adenohypophysis) and a smaller posterior area (neurohypophysis). It sits in a small pocket of bone in the base of the skull called the pituitary fossa, also known as the sella turcica, or Turkish saddle, because of the resemblance. The sella turcica is located deep within the skull, which can be imagined by drawing an imaginary line from the top of the nose through the brain to the back of the head, and from ear to ear. The point at which they intersect is where the pituitary gland sits.

Pituitary disorders can cause a wide spectrum of symptoms, both hormonal and neurological, due to its location near the brain, the intracranial nerves and blood vessels and because of the vital hormonal control that the gland provides. Diagnosis of pituitary disease may be extremely difficult because it's often confused with other disorders. It is dependent on symptoms, signs on examination, blood tests and MRI findings. Direct access to the pituitary can only be reached at surgery.

Thyroid Stimulating Hormone (TSH) causes the thyroid gland to produce and release thyroid hormones. Thyroid hormone controls the basal metabolic rate and plays an important role in growth and maturation. Thyroid hormones affect almost every organ in the body.

Growth Hormone (GH) regulates growth and metabolism. Adrenocorticotropic Hormone (ACTH) triggers the adrenals to release the hormone cortisol, which regulates carbohydrate, fat, and protein metabolism and blood pressure. The adrenal glands sit above the kidneys and are also responsible for the body's fight or flight response.

Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH) control the production of sex hormones (estrogen and testosterone) and sperm and egg maturation and release.

Melanocyte-Stimulating Hormone (MSH) regulates the production of melanin, a dark pigment, by melanocytes in the skin. Increased melanin production produces pigmentation or tanning of the skin; in certain conditions excessive production of melanocyte-stimulating hormone can cause darkening of the skin.

Prolactin (PRL) stimulates production of breast milk and is necessary for normal milk production during breast feeding.

– What happens if the pituitary produces either too much or too little of these hormones?

An imbalance occurs, leading to more than a dozen disorders of the endocrine system. Deficiency of thyroid hormone, adrenal cortical hormone (cortisol) or antidiuretic hormone (vasopressin) is rapidly life-threatening. In patients with abnormalities of the other hormones, quality of life is significantly compromised.

Tumors (overwhelmingly benign), inflammation, infections and injury can cause the gland to malfunction, as well as metastasis or spread of other tumors to the pituitary (rare). Radiation therapy to the brain can also cause normal pituitary cells to malfunction.

A pituitary tumor is an abnormal growth of pituitary cells. Pituitary tumors can either be nonfunctional (that is they do not secrete hormones) or produce specific hormones, such as prolactin (causing infertility, decreased libido, and osteoporosis), growth hormone (causing acromegaly), ACTH (causing Cushing's), TSH (causing hypothyroidism), or be nonfunctional (that is they do not produce hormones). These tumors behave according to their cell of origin and are named for the specific cell type affected. For example, if a tumor originates in a prolactin producing cell, the patient develops a prolactinoma-a prolactin secreting pituitary tumor that is common and usually treatable. High prolactin levels suppress production of the pituitary hormones (luteinizing hormone and follicle stimulating hormone) that stimulate production of estrogen or testosterone. Men with these tumors have low testosterone levels and lose their sex drive and eventually their masculine characteristics-hair, muscle, erections, and ability to produce sperm. Women with prolactin producing tumors often do not ovulate, experience low estrogen levels, and cease having menstrual periods. In both cases, patients with low sex hormones develop osteoporosis. It is important to remember that most pituitary tumors are benign and cancer is very rare. They have variable patterns of growth and affect different people in vastly different ways. Some are small and incidental, while others are small but cause hormone excess. Others may be rapidly growing mass lesions.

If diagnosed early enough, the prognosis is typically excellent. If not, some tumors can grow into macroadenomas that press on the optic nerves (causing loss of peripheral vision and, in some cases, blindness), the brain (impacting function) and the pituitary (limiting or eliminating hormone production). Large tumors can also invade the cavernous sinuses, which house the carotid arteries, the veins to drain the eyes, and nerves involved in eye movement. Surgery around the cavernous sinuses is difficult and should be performed by an experienced pituitary surgeon. The tumors can also secrete too much of a hormone, causing hypertension, diabetes, mood disorders, sexual dysfunction, infertility, osteoporosis, arthritis, accelerated heart disease and death.

The confusing constellation of symptoms that can be produced by pituitary tumors and the difficult to visualize location make diagnosis very tricky. It is not uncommon for patients to have symptoms of either hormonal deficiency (caused by compression of the pituitary or its "stalk") or hormone excess (caused by unregulated production of hormones by the pituitary tumor). In a significant minority of patients diagnosis is not made until the individual has developed debilitating or life-threatening symptoms of heart disease or adrenal (uncommon), gonadal and/or thyroid insufficiency. Even in the 21st century death from a large pituitary tumor or hormonal deficiency still occurs, albeit rarely. Early diagnosis is usually a reflection of a high index of suspicion on the part of a physician. Unfortunately, many doctors have been taught that pituitary disease is rare, so it is not at the forefront of their list of possible diagnoses.

Autopsy reports and radiologic and MRI evidence from around the globe indicate that one out of every five people worldwide has a pituitary tumor. The earliest study took place in 1936, when Dr. R.T. Costello of the Mayo Foundation conducted a cadaver study and found pituitary tumors in 22.4 % of the population (Costello R.T. Subclinical adenoma of the pituitary gland. Am. J. Pathol. 1936; 12:205-214). Statistics have not changed much ever since. The clinical significance of these findings are critical to determine.

We don't know because funding for benign brain tumor research is virtually nonexistent. That's about to change. In October 2002, Congress passed the Benign Brain Tumor Cancer Registries Amendment Act, which will force hospitals, clinics and doctors to report pituitary tumor incidence rates in the data collection of cancer registries. The problem remains diagnosis. No report of incidence rates is possible without it.

Pituitary tumors/disease present a vast array of symptoms, and it's often the symptoms that get treated, not the disease. As a result, pituitary patients can spend years being misdiagnosed as their tumors grow. People with undetected pituitary tumors can die of heart attacks, hypothyroidism, adrenal insufficiency or water balance problems, all of which can mask the main cause: a pituitary tumor.

Dollars spent. As a result, we have failed to answer the most important question: Why are pituitary tumors so common?

There is a lack of education within the medical community and among the general public.

The insurance industry hasn't caught on to the untold billions of dollars that could be saved through early diagnosis and treatment. Once it becomes clear that it's in everyone's best interest, the word will spread.

It depends on the type of tumor and how far it has invaded into the brain, as well as the patient's age and overall health. Three kinds of treatment are used: surgery (removing the tumor during an operation), radiation therapy (using high-dose x-rays/proton beams to kill tumor cells) and drug therapy to shrink and sometimes eradicate the tumor. Drugs can also block the pituitary gland from making too much hormone. A Transphenoidal operation can remove the tumor through a cut in the nasal passage, leaving no external scar. (Sometimes a surgeon prefers to go through an incision under the upper lip instead of the nose, also with no external scarring.) A craniotomy removes the tumor through a cut in the front of the skull; this is rarely done for large invasive tumors.

Surgery is a common treatment, and it is almost always successful IF performed by a skilled and specialized neurosurgeon. The smaller the tumor, the greater the chance the surgery will be successful. Large tumors that grow upward (into the nerves that mediate vision) or laterally (into the cavernous sinuses that contain the major arteries to the brain-the carotid arteries) increase the risk of surgery and are generally associated with a lower probability of cure. Early diagnosis and treatment is the key.

Radiation therapy uses high-energy x-rays to kill cancer cells and abnormal pituitary cells and shrink tumors. Radiation for pituitary tumors these days almost always comes from a radiation source outside the body (external radiation therapy). Radiation therapy may be used if medication/surgery fails to control the tumor. There are several different types of radiation therapy.

Standard External Beam Radiotherapy uses a radiation source that is nonselective and radiates all cells in the path of the beam; in almost all cases other portions of the brain in the area of the pituitary are in the path of the radiation beam. Proton Beam Treatment employs a specific type of radiation in which "protons", a form of radioactivity, are directed specifically to the pituitary gland. The advantage of this technique is less damage to tissues surrounding the pituitary gland.

Gamma Knife Irradiation combines standard external beam radiotherapy with a technique that focuses the radiation through many different ports. This treatment tends to do less damage to tissues adjacent to the pituitary gland. Some clinics are using proton beam therapy; laser-guided MR imaged stereotactic intrasellar tumor localization can be used for selected patients with untreated pituitary tumors, incomplete surgical resection, or tumor recurrence.

No. There are more than a dozen very different disorders that result from pituitary tumors and disease. For example, if a tumor forms in an ACTH secreting pituitary cell, it could result in the overproduction of cortisol (Cushing's Disease) or the underproduction of cortisol (adrenal insufficiency, often referred to as Secondary Addison's Disease). Cushing's is a condition characterized by excessive fat accumulation in central parts of the body (obesity, including a rounded or fat-filled face), diabetes, hypertension, a low serum potassium, thinning and bruising of skin, and osteoporosis. Symptoms of adrenal insufficiency include dehydration, low blood pressure and sodium level, and excessive weight loss. Primary Addison's Disease is caused when the adrenal glands fail to work on their own, i.e. fail to respond to directions from the pituitary and hypothalamus.

If the tumor forms in a growth hormone producing cell, it can overproduce growth hormone. Tumors that form from growth hormone producing cells cause two different clinical pictures. If they occur in children before the growth plates in long bones have closed, excessive growth hormone will cause gigantism. (Do you recall the movie "My Giant" with Billy Crystal and the basketball player?). If the growth hormone excess occurs during adulthood there is excessive enlargement of the hands, feet, and jaw, as well as soft tissue swelling of many tissues (acromegaly). Acromegaly is associated with an increased probability of developing diabetes mellitus, heart attack, hypertension, and certain types of cancer including malignancy of the colon. Most commonly the facial changes develop subtly and may not be noticed by the patient or his/her family.

There may be permanent loss of some or all pituitary hormones, an imbalance that can be treated with Hormone Replacement Therapy, which has been inaccurately associated with only one group of patients: post-menopausal women. HRT can replace thyroid, growth, testosterone, or adrenal hormones when those made by the pituitary to stimulate the endocrine glands are no longer produced. It can be lifesaving therapy for the many millions of patients who need to replace hormones they no longer make.

Anyone suffering from a constellation of complaints/clinical findings suspicious for pituitary disease should get blood and urinary hormone levels checked and, if indicated, a brain MRI, keeping in mind that microadenomas may not show up on the x-rays. Combinations of three or more of the following may suggest the possibility of a pituitary tumor: sexual dysfunction, depression, galactorrhea, infertility, growth problems, osteoporosis, obesity (specifically central), severe vision problems, easy bruising, aching joints, carpel tunnel syndrome, disrupted menses, early menopause, muscle weakness, fatigue.

It is important to go to a hospital with a Neuroendocrine Unit or Pituitary Testing Facility, preferably both. (This may necessitate plane travel, but it would be worth every penny.) Pituitary blood tests can be very complicated and must be performed by specially trained technicians who can conduct dynamic hormone testing and precisely-timed blood sampling, administer testing agents, and have special expertise in measuring pituitary hormones. Tests that require special attention include ACTH (Cortrosyn) stimulation, bilateral inferior petrosal sinus ACTH sampling, Insulin tolerance, growth hormone stimulation, TRH stimulation, GnRH stimulation, Oral glucose tolerance, and GHRH/arginine testing for growth hormone reserve.

Ideally you want to be seen by a pituitary endocrinologist associated with a neuroendocrine or pituitary unit with a pituitary testing facility. The interpretation of complex blood tests can result in misdiagnoses. This can be associated with microadenomas (very small tumors) that do not show up on an MRI of the brain. It often takes a real expert to get the right answers and avoid pitfalls.

Dr. Edward Laws is not my neurosurgeon. My neurosurgeon trained directly with Dr. Laws. Dr. Laws is widely considered to be the best pituitary surgeon in the world. He has done more pituitary surgeries than anyone else. Like over 4,500.

My neurosurgeon was the chief neurosurgery resident under Dr. Laws during his tenure at the University of Virginia. They would do 6 pituitary surgeries a day together.

In surgery, it is all about who you train with. I have complete faith in my doctor. I really felt positive about my surgeon choice before researching Laws again. Now, I admit to feeling even better!

Photo by Andrew ShurtleffDr. Edward Laws prepares to remove a pituitary tumor from a patient. These tumors account for 20 percent of all primary brain tumors, and Laws is internationally recognized as the top pituitary surgeon.

By Fariss Samarrai

Dr. Edward Laws stands on a wooden pedestal as he extracts a brain tumor through a woman’s right nostril. His resident, Dr. Adam Kanter, and a team of operating room specialists, have prepared the patient for the procedure by first anesthetizing her, and then, by sending long and slim instruments up the woman’s nostril, they have removed a thin wafer of bone, allowing direct access through the air sinuses to the pituitary gland at the base of the brain.

Laws’ job is to remove a tumor that has grown on the pituitary by carefully scraping loose and periodically removing fragments of the mass of bad cells while being careful to leave the healthy tissue of the pituitary and the brain intact. He does this while looking at the inside of the patient’s head through a binocular endoscope with a micro-camera attached, allowing everyone in the OR to observe the procedure on a video monitor.

He pulls out another fragment of the tumor.

“These tumors have the texture of tofu,” he says, though the tissue he is extracting is bloody. He mentions in passing that he used to tell people that pituitary tumors have the texture of tapioca, but that many people don’t seem to know what that is anymore.

Laws removes a final fragment of the tumor, and his work is done for now. The procedure is called trans-sphenoidal surgery — basically access-through-the-nose surgery. By day’s end Laws will have removed pituitary tumors from six patients, likely making the lives of these people immeasurably better.

Laws is director of the Pituitary Center at U.Va. He has removed pituitary tumors from more than 4,300 patients, more than any other doctor in the world. Most of these surgeries have been performed at the U.Va. center he helped found in 1972. Laws is internationally recognized as the top pituitary tumor surgeon. Medical residents from around the world come to U.Va. to train under him.

Illustration by Craig Luce

Pituitary TumorsWhere they are and how they are removedThe pituitary gland is located at the base of the brain near the spinal cord, in the lower center of the head. This gland, normally the size of a pea, is the master gland that controls the production of all hormones. The pituitary controls growth, sexual maturation, fertility, metabolism, stress response and fluid balances. Pituitary tumors are reached by passing long and slim surgical instruments through the air sinuses to the diseased gland.

The procedure, like all planned operations, is methodical, calm, controlled, predictable, with precautions in place for possible problems or emergencies. The operation is so un-invasive, it more resembles routine dental work than major surgery. Yet, Laws and his colleagues are working at the very edge of the brain. There is little room here for error.

In past years, pituitary surgery was more dramatic, and intensive, for both the surgeon and the patient. It involved removing a portion of the skull and pushing the brain aside to reach the pituitary. The recovery period was long, and there was much greater risk for damage and complications. Laws and others invented and refined the current technique — passage through the nose — which rarely involves direct contact with the brain, even as they operate within its midst.

The pituitary gland is located at the base of the brain near the spinal cord, in the lower center of the head. This gland, normally the size of a pea, is the master gland that controls the production of all hormones. The pituitary controls growth, sexual maturation, fertility, metabolism, stress response and fluid balances.

For reasons not well understood, the pituitary is subject to the slow growth of tumors. These tumors are the third-most common type of primary brain tumor, accounting for 20 percent of all brain tumors. Though these tumors are almost always benign, they aren’t friendly. They can cause the pituitary gland to produce excessive or insufficient amounts of hormones, leading to a vast array of unpleasant symptoms, including severe and chronic headaches, infertility, sexual dysfunction, fatigue, weight gain, depression, apathy, sleep disorders, memory loss, and in some cases, loss of vision. Pituitary disorders also are the cause of Cushing’s syndrome, which results in obesity, “moonlike” features of the face, excess body hair and thin brittle bones.

Pituitary surgeries are discussed prior to and after each operation.

Because the symptoms of pituitary disorders are so varied, it can take years to arrive at a correct diagnosis,” Laws said. “Patients often think that their health problems are attributed to just getting older.”

It takes a brain scan in the form of magnetic resonance imaging to confirm the presence of a pituitary tumor. Sometimes the tumor is three times the size of the gland itself, putting pressure on the brain and optic nerve, resulting in headaches and vision loss. Small tumors often can be treated with hormones and drugs. Radiation may shrink the tumor or inhibit its growth. If the tumor continues to grow, and symptoms worsen, surgery may be necessary.

Laws discovered early, at the beginning of his medical training at Johns Hopkins University, that he was interested in neurosurgery. His fascination with the brain has continued throughout his 40-year career as a surgeon and brain tumor researcher.“How beautiful the brain is,” he said recently. “I’m amazed with what happens with the human brain, and I love dealing with it in an intimate fashion.” He describes the brain as “one of the greatest of God’s creations.”

But he’s happy to fix that creation when something goes wrong.

“We can restore vision,” he said of pituitary surgery. “We can restore fertility and reverse body changes. This is enormously satisfying.”

These tumors are identified using magnetic resonance imaging.

One of the first patients Laws treated with the through-the-nose surgical method was a 21-year-old man named Harvey Gartner. The surgery was performed in 1969 at The Johns Hopkins Hospital where Laws was a neurosurgery resident. Gartner had been growing rapidly since he was 6 months old. His bones were elongated, and he suffered from arthritis and other ailments.

“Those were tough years,” Gartner said of his youth.

The operation took 12 hours to complete, and all of the pituitary gland was removed, as was standard at the time. Gartner, now 56, is on lifetime hormone replacement therapy, but his health is generally good.

“I would have died,” if not for the surgery, he said.

Gartner is now a computer programmer and businessman in Jacksonville, Fla. He has remained in contact with Laws, and the pediatrician who cared for him throughout his life, Dr. Robert Blizzard, a U.Va. professor emeritus of pediatric endocrinology. At the time of Gartner’s surgery, Hopkins was the top pituitary center in the United States. That title has long since shifted to U.Va. as a result of Laws, Blizzard and others coming here to establish their interdisciplinary center.

Today, surgeons rarely need to remove the entire pituitary gland. Instead, when surgery is necessary, they remove only the tumor attached to the gland. The entire procedure, including prepping the patient for surgery and the post-operative period, can be accomplished in a couple of hours. The patient is able to return home in two or three days and can resume normal activities in two to four weeks.

Within a few days after surgery, the patient’s severe headaches will have diminished. Vision will be restored within a day in cases where the tumor had pressed against the optic nerve. Body changes, such as weight loss and normalization of facial features, will occur in about six months. Throughout the patient’s life, teams of endocrinologists and other specialists will measure the patient’s hormone levels and develop and refine treatment plans. Laws said some patients might not even need hormone replacement therapy.

“I don’t think anybody does this work better than we do at U.Va.,” Laws said recently between surgeries. “We’re improving our techniques and reducing the recurrence of tumors after surgery.”

At this moment he is called back into the operating room. Another patient is prepped and waiting.

I've finally been diagnosed and am going to have a transsphenoidal. What can I expect post-surgery?

AFTER YOUR TRANSSPHENOIDAL SURGERY

After your physicians have determined that it is reasonably safe to discharge you from the hospital following transsphenoidal pituitary surgery there are a number of important situations that may arise. Most people feel well after discharge. However, you should be aware of these possible problems, just in case. The following general guidelines are provided to promote your health and safety. Headache, facial, and sinus pain are not uncommon following pituitary surgery. As you may have noted, the pain and discomfort typically improve on a daily basis following surgery. If you should experience a worsening of your pain or discomfort, please contact your neurosurgeon immediately.

Worsening headache, fever, chills, yellowish green nasal discharge, and neck stiffness may all signify an infectious process complicating your surgery. You should notify either your neurosurgeon, endocrinologist, or primary physician immediately should any of these symptoms and signs develop.

Persistent bloody, clear watery, or yellowish green nasal discharge should prompt an immediate call to one of your physicians.

Development of abnormalities in your vision should prompt an urgent call to your neurosurgeon, neuroopthalmologist, or any other one of your physicians.

Chest pain or discomfort, shortness of breath, swelling of one or both of your legs, and passage of dark black tarry stools may represent medical complications in patients who undergo surgery of any type. Contact your physicians should any of these symptoms or signs occur.

Some patients develop disorders of salt and water metabolism following pituitary surgery. Headache, nausea, vomiting, confusion, impaired concentration, and muscle aches might be due to hyponatremia (low blood sodium levels). This disorder typically occurs 7 to 10 days after surgery and is more common in patients who have had surgery for Cushing's disease. If you develop these symptoms, contact your endocrinologist or one of your other physicians immediately. Excessive urination, thirst, and the need to ingest large quantities of fluids might be related to the onset of diabetes insipidus or diabetes mellitus. These disorders put you at risk for dehydration. The symptoms require urgent evaluation and determination of the underlying cause so that appropriate treatment may be given. Thus, if these symptoms develop, contact your endocrinologist or one of your other physicians immediately.

You may or may not have been prescribed hormones at the time of discharge. If so, you should take these medications, without interruption, as prescribed by your physician. Adjustments in your glucocorticoid hormone dosage may be required. Please consult the instructions for patients with adrenal insufficiency for general recommendations. You may be asked to withhold your dose of glucocorticoid replacement at the time of your first postoperative follow-up visit. Contact your endocrinologist for advice on this matter if specific instructions have not been provided. The instructions for patients with hypothyroidism on thyroxine replacement may be consulted for advice regarding thyroid hormone medication. You should consult the instructions for patients with diabetes insipidus treated with vasopressin if you have been diagnosed with diabetes insipidus or suspect that you may have developed the disorder. Above all, contact your physicians if you have any questions whatsoever about any one of your medications.

In general, the first postoperative follow-up visit will be scheduled to occur four weeks after surgery. If problems develop prior to that time, you will be asked to return to the office for evaluation. Subsequent follow-up is tailored to the individual needs of each patient and in part depends upon the diagnosis, presence of residual disease, likelihood of recurrent disease, extent and type of hormonal disorders, and other complications of pituitary disease.

In most cases, lifelong follow-up is necessary. You should ensure that you receive appropriate follow-up by physicians knowledgeable regarding the diagnosis and management of pituitary disorders.

********

Once again, MaryO has done a wonderful job organizing the Cushing's Help and Support website to help us Cushies access info when we need it. Thanks, Mary!

NOTE: Adrenal insufficiency is not one of those risks from surgery that may happen. In the hundreds of people I have watched have pituitary surgery in the past few years, nearly EVERYONE has gone to the hospital with adrenal insufficiency within the first six months after surgery. Many go multiple times. It is not only possible, it is probable.

For women, imagine the changes in your body during menstruation. Body aches, depression, cramping, irritability, fatigue, "drained." Well, that results from only a few hormones moving up and down, and they only affect you for a few days, maybe a week.

With Cushing's and the adrenal insufficiency we face after pituitary surgery, all of our hormones are going nutzo. Every single one of them. We suffer from the symptoms above, and those outlined below. Oh, and it doesn't last days. It lasts months and years.

Add to the mix this fun little fact: no one around you--not your local friends or family--have ever seen this happen to anyone.

OverviewAddisons disease is a rare endocrine, or hormonal disorder that affects about 1 in 100,000 people. It occurs in all age groups and afflicts men and women equally. The disease is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and sometimes darkening of the skin in both exposed and non-exposed parts of the body.

Addisons disease occurs when the adrenal glands do not produce enough of the hormone cortisol and in some cases, the hormone aldosterone. For this reason, the disease is sometimes called chronic adrenal insufficiency, or hypocortisolism.

Cortisol is normally produced by the adrenal glands, located just above the kidneys. It belongs to a class of hormones called glucocorticoids, which affect almost every organ and tissue in the body. Scientists think that cortisol has possibly hundreds of effects in the body. Cortisol's most important job is to help the body respond to stress. Among its other vital tasks, cortisol:

Because cortisol is so vital to health, the amount of cortisol produced by the adrenal's is precisely balanced. Like many other hormones, cortisol is regulated by the brains hypothalamus and the pituitary gland, a bean-sized organ at the base of the brain. First, the hypothalamus sends "releasing hormones" to the pituitary gland. The pituitary responds by secreting other hormones that regulate growth, thyroid and adrenal function, and sex hormones such as estrogen and testosterone. One of the pituitarys main functions is to secrete ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands. When the adrenals receive the pituitarys signal in the form of ACTH, they respond by producing cortisol. Completing the cycle, cortisol then signals the pituitary to lower secretion of ACTH.

Aldosterone belongs to a class of hormones called mineralocorticoids, also produced by the adrenal glands. It helps maintain blood pressure and water and salt balance in the body by helping the kidney retain sodium and excrete potassium. When aldosterone production falls too low, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop.

Causes

Failure to produce adequate levels of cortisol, or adrenal insufficiency, can occur for different reasons. The problem may be due to a disorder of the adrenal glands themselves (primary adrenal insufficiency) or to inadequate secretion of ACTH by the pituitary gland (secondary adrenal insufficiency).

Primary Adrenal Insufficiency

Most cases of Addisons disease are caused by the gradual destruction of the adrenal cortex, the outer layer of the adrenal glands, by the body's own immune system. About 70 percent of reported cases of Addisons disease are due to autoimmune disorders, in which the immune system makes antibodies that attack the body's own tissues or organs and slowly destroy them. Adrenal insufficiency occurs when at least 90 percent of the adrenal cortex has been destroyed. As a result, often both glucocorticoid and mineralocorticoid hormones are lacking. Sometimes only the adrenal gland is affected, as in idiopathic adrenal insufficiency; sometimes other glands also are affected, as in the polyendocrine deficiency syndrome.

The polyendocrine deficiency syndrome is classified into two separate forms, referred to as type I and type II. Type I occurs in children, and adrenal insufficiency may be accompanied by underactive parathyroid glands, slow sexual development, pernicious anemia, chronic candida infections, chronic active hepatitis, and, in very rare cases, hair loss. Type II, often called Schmidts syndrome, usually afflicts young adults. Features of type II may include an underactive thyroid gland, slow sexual development, and diabetes mellitus. About 10 percent of patients with type II have vitiligo, or loss of pigment, on areas of the skin. Scientists think that the polyendocrine deficiency syndrome is inherited because frequently more than one family member tends to have one or more endocrine deficiencies.

Tuberculosis (TB) accounts for about 20 percent of cases of primary adrenal insufficiency in developed countries. When adrenal insufficiency was first identified by Dr. Thomas Addison in 1849, TB was found at autopsy in 70 to 90 percent of cases. As the treatment for TB improved, however, the incidence of adrenal insufficiency due to TB of the adrenal glands has greatly decreased.

Less common causes of primary adrenal insufficiency are chronic infections, mainly fungal infections; cancer cells spreading from other parts of the body to the adrenal glands; amyloidosis; and surgical removal of the adrenal glands. Each of these causes is discussed in more detail below.

Secondary Adrenal Insufficiency

This form of Addisons disease can be traced to a lack of ACTH, which causes a drop in the adrenal glands production of cortisol but not aldosterone. A temporary form of secondary adrenal insufficiency may occur when a person who has been receiving a glucocorticoid hormone such as prednisone for a long time abruptly stops or interrupts taking the medication. Glucocorticoid hormones, which are often used to treat inflammatory illnesses like rheumatoid arthritis, asthma, or ulcerative colitis, block the release of both corticotropin-releasing hormone (CRH) and ACTH. Normally, CRH instructs the pituitary gland to release ACTH. If CRH levels drop, the pituitary is not stimulated to release ACTH, and the adrenals then fail to secrete sufficient levels of cortisol.

Another cause of secondary adrenal insufficiency is the surgical removal of benign, or noncancerous, ACTH-producing tumors of the pituitary gland (Cushings disease). In this case, the source of ACTH is suddenly removed, and replacement hormone must be taken until normal ACTH and cortisol production resumes. Less commonly, adrenal insufficiency occurs when the pituitary gland either decreases in size or stops producing ACTH. This can result from tumors or infections of the area, loss of blood flow to the pituitary, radiation for the treatment of pituitary tumors, or surgical removal of parts of the hypothalamus or the pituitary gland during neurosurgery of these areas.

SYMPTOMS

The symptoms of adrenal insufficiency usually begin gradually. Chronic, worsening fatigue and muscle weakness, loss of appetite, and weight loss are characteristic of the disease. Nausea, vomiting, and diarrhea occur in about 50 percent of cases. Blood pressure is low and falls further when standing, causing dizziness or fainting. Skin changes also are common in Addisons disease, with areas of hyperpigmentation, or dark tanning, covering exposed and nonexposed parts of the body. This darkening of the skin is most visible on scars; skin folds; pressure points such as the elbows, knees, knuckles, and toes; lips; and mucous membranes.

Addisons disease can cause irritability and depression. Because of salt loss, craving of salty foods also is common. Hypoglycemia, or low blood sugar, is more severe in children than in adults. In women, menstrual periods may become irregular or stop.

Because the symptoms progress slowly, they are usually ignored until a stressful event like an illness or an accident causes them to become worse. This is called an addisonian crisis, or acute adrenal insufficiency. In most patients, symptoms are severe enough to seek medical treatment before a crisis occurs. However, in about 25 percent of patients, symptoms first appear during an addisonian crisis.

Symptoms of an addisonian crisis include sudden penetrating pain in the lower back, abdomen, or legs; severe vomiting and diarrhea, followed by dehydration; low blood pressure; and loss of consciousness. Left untreated, an addisonian crisis can be fatal.

DIAGNOSIS

In its early stages, adrenal insufficiency can be difficult to diagnose. A review of a patients medical history based on the symptoms, especially the dark tanning of the skin, will lead a doctor to suspect Addisons disease.

A diagnosis of Addisons disease is made by biochemical laboratory tests. The aim of these tests is first to determine whether there are insufficient levels of cortisol and then to establish the cause. X-ray exams of the adrenal and pituitary glands also are useful in helping to establish the cause.

ACTH Stimulation Test

This is the most specific test for diagnosing Addisons disease. In this test, blood and/or urine cortisol levels are measured before and after a synthetic form of ACTH is given by injection. In the so called short, or rapid, ACTH test, cortisol measurement in blood is repeated 30 to 60 minutes after an intravenous ACTH injection. The normal response after an injection of ACTH is a rise in blood and urine cortisol levels. Patients with either form of adrenal insufficiency respond poorly or do not respond at all.

When the response to the short ACTH test is abnormal, a "long" ACTH stimulation test is required to determine the cause of adrenal insufficiency. In this test, synthetic ACTH is injected either intravenously or intramuscularly over a 48- to 72-hour period, and blood and/or urine cortisol are measured the day before and during the 2 to 3 days of the injection. Patients with primary adrenal insufficiency do not produce cortisol during the 48- to 72-hour period; however, patients with secondary adrenal insufficiency have adequate responses to the test on the second or third day.

In patients suspected of having an addisonian crisis, the doctor must begin treatment with injections of salt, fluids, and glucocorticoid hormones immediately. Although a reliable diagnosis is not possible while the patient is being treated, measurement of blood ACTH and cortisol during the crisis and before glucocorticoids are given is sufficient to make the diagnosis. Once the crisis is controlled and medication has been stopped, the doctor will delay further testing for up to 1 month to obtain an accurate diagnosis.

Insulin-Induced Hypoglycemia Test

A reliable test to determine how the hypothalamus and pituitary and adrenal glands respond to stress is the insulin-induced hypoglycemia test. In this test, blood is drawn to measure the blood glucose and cortisol levels, followed by an injection of fast-acting insulin. Blood glucose and cortisol levels are measured again at 30, 45, and 90 minutes after the insulin injection. The normal response is for blood glucose levels to fall and cortisol levels to rise.

Other Tests

Once a diagnosis of primary adrenal insufficiency has been made, x-ray exams of the abdomen may be taken to see if the adrenals have any signs of calcium deposits. Calcium deposits may indicate TB. A tuberculin skin test also may be used.

If secondary adrenal insufficiency is the cause, doctors may use different imaging tools to reveal the size and shape of the pituitary gland. The most common is the CT scan, which produces a series of x-ray pictures giving a cross-sectional image of a body part. The function of the pituitary and its ability to produce other hormones also are tested.

TREATMENT

Treatment of Addisons disease involves replacing, or substituting, the hormones that the adrenal glands are not making. Cortisol is replaced orally with hydrocortisone tablets, a synthetic glucocorticoid, taken once or twice a day. If aldosterone is also deficient, it is replaced with oral doses of a mineralocorticoid, called fludrocortisone acetate (Florinef)), which is taken once a day. Patients receiving aldosterone replacement therapy are usually advised by a doctor to increase their salt intake. Because patients with secondary adrenal insufficiency normally maintain aldosterone production, they do not require aldosterone replacement therapy. The doses of each of these medications are adjusted to meet the needs of individual patients.

During an addisonian crisis, low blood pressure, low blood sugar, and high levels of potassium can be life threatening. Standard therapy involves intravenous injections of hydrocortisone, saline (salt water), and dextrose (sugar). This treatment usually brings rapid improvement. When the patient can take fluids and medications by mouth, the amount of hydrocortisone is decreased until a maintenance dose is achieved. If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate.

SPECIAL PROBLEMS

Surgery

Patients with chronic adrenal insufficiency who need surgery with general anesthesia are treated with injections of hydrocortisone and saline. Injections begin on the evening before surgery and continue until the patient is fully awake and able to take medication by mouth. The dosage is adjusted until the maintenance dosage given before surgery is reached.

Pregnancy

Women with primary adrenal insufficiency who become pregnant are treated with standard replacement therapy. If nausea and vomiting in early pregnancy interfere with oral medication, injections of the hormone may be necessary. During delivery, treatment is similar to that of patients needing surgery; following delivery, the dose is gradually tapered and the usual maintenance doses of hydrocortisone and fludrocortisone acetate by mouth are not reached until about 10 days after childbirth.

Patient Education

A person who has adrenal insufficiency should always carry identification stating his or her condition in case of an emergency. The card should alert emergency personnel about the need to inject 100 mg of cortisol if its bearer is found severely injured or unable to answer questions. The card should also include the doctors name and telephone number and the name and telephone number of the nearest relative to be notified. When traveling, it is important to have a needle, syringe, and an injectable form of cortisol for emergencies. A person with Addisons disease also should know how to increase medication during periods of stress or mild upper respiratory infections. Immediate medical attention is needed when severe infections or vomiting or diarrhea occur. These conditions can precipitate an addisonian crisis. A patient who is vomiting may require injections of hydrocortisone.

It is very helpful for persons with medical problems to wear a descriptive warning bracelet or neck chain to alert emergency personnel. Bracelets and neck chains can be obtained from:

This epub was written by Eileen K. Corrigan of NIDDKs Office of Health Research Reports. The draft was reviewed by Dr. George P Chrousos, National Institute of Child Health and Human Development, Dr. Judith Fradkin, National Institute of Diabetes and Digestive and Kidney Diseases, and by Dr. Richard Horton, University of Southern California Medical Center.

This epub is not copyrighted. Readers are encouraged to duplicate and distribute as many copies as needed. Printed single copies may be obtained from the Office of Health Research Reports, NIDDK, Building 31, Room 9A04, Bethesda, Maryland 20892.

NIH Publication No. 90-3054

*************

This is the Adrenal Crisis publication distributed with the letter from Cushing's Research and Support Foundation to family and friends.

Adrenal Insufficiency & Cortisol Withdrawal

After pituitary and adrenal surgery, adrenal insufficiency can occur as replacement steroid hormones are tapered and adrenal hormone production slowly improves toward normal. If hormone production does not return to normal then replacement hormones will be needed on a permanent basis.

If you have pituitary or adrenal surgery it is recommended that you wear an ID bracelet, or necklace that will identify you as being adrenal insufficient. There are many places to purchase medical ID's. Your local jewelry store or pharmacy will probably have them. There's also a vast supply of merchants that sell medical ID's on the Internet. (I purchased mine through

With Cushing's the body is producing too much cortisol. Over a period of time the body gets accustomed to the high levels of cortisol.

After surgery for Cushing's, ideally the cortisol level will decline. As the decline in cortisol level continues, it is common to experience withdrawal. The pain from withdrawal can be horrible but it's a positive sign that you likely got a cure from the Cushing's through surgery. It is important that you follow your doctor's advice as he will adjust your replacement dosage accordingly.

It's important to KNOW the difference between what is considered to be common cortisol withdrawal symptoms and Adrenal Insufficiency. Death can occur from Adrenal Insufficiency due to overwhelming shock if early treatment is not provided. Always follow your doctor's advice and never adjust your replacement medications on your own.

Adrenal Insufficiency is an abrupt, life-threatening state caused by insufficient cortisol, a hormone produced and released by the adrenal gland.

Those who are at risk for Adrenal Insufficiency should be taught to recognize signs of potential stress that may precipitate an acute adrenal crisis. An Adrenal Crisis can occur suddenly and unexpectedly. Some people are taught to give themselves an emergency injection of hydrocortisone in times of stress. It is important for the individual to always carry a medical identification card that states the type of medication and the proper dose needed in case of an emergency. Never omit replacement medication. If unable to retain medication due to vomiting, notify the health care provider as you may need injectable replacement.

Some of the initial symptoms of Adrenal Insufficiency may include but not limited to some of the following symptoms headache, weakness, fatigue, nausea and/or vomiting, abdominal cramps, diarrhea, confusion, rapid heart rate, joint pain, chills, fever, low blood pressure, etc.

Later symptoms, as the Adrenal Insufficiency progresses, may include but not limited to dehydration, darkening of the skin, weight loss, appetite loss, rash, and coma.

Treatment:Intravenous or intramuscular injection of hydrocortisone (injectable corticosteroid) must be given immediately. Treatment of low blood pressure is usually necessary and may be treated with intravenous fluids. Hospitalization is often required for adequate treatment and monitoring.

It's very important for someone who has the potential to have Adrenal Insufficiency to let their family members and friends know what the symptoms are and what actions should be taken in an emergency situation.

Total Pageviews

Discovery Health's Mystery Diagnosis

When illness strikes, we look to doctors for answers. What if they can't help? For these unlucky patients, diagnosis is a mystery.

Old and New Me

In 2014, i'm reversing my cortisol in search of my old self

Disclaimer: I Am Not a Doctor

I am not a medical professional. I am not a doctor nor have I gone to med school. I am not a physician assistant, a nurse, or a lab technician. I do not currently work in the medical field.

Perhaps what I can provide is actually better and more useful: a tale of odd symptoms, mild physical deformities, and emotional/mental experiences that match your own. It's not just you! I can share my experience in the diagnosis and treatment of my Cushing's disease. Due to this disease's 'rarity,' I relied on my Cushie friends to guide me as we fight this terrible disease. We all quickly learned the basics and the jargon: the body parts, hormone loops, testing protocals, surgical stats, surgeon bios and pedigrees, and paths to recovery. We have seen patients have successful surgeries, and we have seen patients continue to suffer after multiple surgeries. We watch the story of Cushing's unfold for our friends in real time. Our friends are case studies that are unimpressed by whether something meets the standard of 'statistically significant' to be included in the medical literature. We Cushies see and hear it all. We encourage you to grow the same eyes and ears.

We urge you to take an active role in your health care and work closely with medical professionals. If you find a doctor dismissive, find a new one. Use my blog to supplement your knowledge base but never substitute my judgment or decisions for your own.

And please, don't sue me. I have no money left over after medical expenses. One day, I swear, I'm gonna take a nice vacation.

In Memorium: Those We Lost to Cushing's (hyperlinks take you to obituaries)

WHERE TO START IF YOU ARE NEW?

This side bar is intended to be a mini Cushings 101. You will find much of what you need to know here. Click through these links and watch these videos. They are my favorites after researching for Cushing's for six full years now. I started this blog in 2008, so the broad view posts about me can be found there. Click around previous blog posts and read, read, read. You will get scared and overwhelmed, but with information, you will start to take charge of your health and you will arm yourself with what you need to win this war. Because you will, you know. You will win this war.

Looking for something? (under construction)

~BEST ADVICE BASED ON MY EXPERIENCE~

This disease is an enigma - a mystery wrapped in a riddle.

Remove the guesswork and pressure off yourself to know how cortisol ravages your body as it swings from high to low to high levels. Very few Cushies when their cortisol levels are high versus low, and frankly, it is quite unfair that we should have to know. Until someone develops a cortisolometer, similar to a glucometer for testing blood sugar, a Cushing's patient may never know for sure whether the cortisol is high or low. I hope the Addison's community will take the lead on that!

I encourage you to test your cortisol testing every night in a row until you get your highs. Do not skip.1) Do 24 hour UFCs with 17-OHS.2) Get cortisol blood draws at midnight and again 30 minutes later.3) Chew a salivary cortisol test swab while getting your blood drawn each time.

I got my biggest diagnostic highs doing this, after 4 years of trying to follow doctors' suggestions of tracking symptoms and only testing once a night around midnight. Take control. Your mission is to show up every night, and hang in there.

TRUST YOUR GUT. It just might save your life.

I am a cyclical Cushing's patient. I had transsphenoidal pituitary brain surgery on June 11, 2009. By going through my nose with scopes and scalpels, my neurosurgeon removed a 3 millimeter ACTH-secreting benign tumor on the left pituitary that disrupted every hormone and every body system. He says the tumor's cytology was consistent with other Cushing's tumors he has removed in the past, meaning that he removed a milky white substance that was different looking that the normal pinkish pituitary gland cells. Within 3 months of my surgery, I knew I was not cured. I still had Cushing's symptoms, and I tested again for a re-occurrence of Cushing's. In 2010 alone, F*I*V*E endocrinologists told me I don't have Cushing's and they think I never did. They said I had pituitary surgery for nothing. That includes two that I saw before my pituitary surgery. It seems doctors really don't know what to do with a Cushing's patient, much less one that comes back still sick after pituitary surgery.

In February 2011, I tested again at my local hospital, and I hit the jackpot: the highest results for midnight cortisol serums and midnight cortisol salivas that I have had in nearly 4 years. I also had nine straight days of high 17-OHS (used to measure urinary cortisol in patients with mild or cyclical Cushing's). I had diagnostic results on three types of cortisol tests: midnight salivary cortisol, midnight cortisol serum, urinary 17-OHS.

I had a 2nd pituitary surgery on April 20, 2011. The neurosurgeon removed a 5-millimeter benign tumor from the right side of my pituitary. I still suffer from high cortisol and high ACTH, with the latter pinpointing the source as pituitary. No tumor shows on my MRI so in August 2012 I started a medical therapy by taking a well-established medication called ketoconazole. This has helped me reverse some but not all of the Cushing's symptoms. Keto can cause liver damage so I must have liver function tests every three months. I hope I can continue to tolerate this medication until a tumor shows. Next MRI scheduled for June 2013.

WHAT DID I LEARN?Rather than test once in a while, I tested every day. My results clearly showed I had an abnormal cortisol production pattern. I was right. Those doctors were wrong. ALL OF THEM WERE WRONG. Trust your gut. You know your body.

Cushing's Awareness Challenge

What Doctors Will Claim You Have Instead of Cushing's:

Fibromyalgia (no lab tests can prove this; diagnosis given when they don't know what else is wrong with you)

Back and muscle pain

Diabetes

Blurred vision, visual field loss, double vision

Chronic fatigue syndrome

Dry, oily, or sweaty skin

Impotence or infertility

Joint pain, joint/bone abnormalities

Migraines

Muscle weakness, carpal tunnel syndrome

Temporomandibular joint/TMJ/jaw joint pain

Thyroid imbalance or "slow metabolism"

Spreading Awareness to the Health Community

Is it possible you have many conditions damaging your body simultaneously? Sure. Is it more likely that you have ONE condition that explains all that ails you? Yes. That could be Cushing's.

In medical school, doctors are trained that it is better to find one diagnosis to fit all symptoms. Go ahead and ask your doctor about that when s/he tells you that you have multiple overlapping illnesses.

Once you get abnormally high biochemical evidence (ACTH and cortisol) in the form of urine, saliva, and/or blood tests, it is highly probable you have Cushing's. Trust your instincts. Don't let the doctors tell you differently.

Different Doctors Treat Symptoms of Cushing's: A List of Professionals You May Have Seen

The pituitary controls the production of every hormone in the body. We Cushies go undiagnosed as we bounce around from medical subfield to subfield. Each doc treats the symptoms they know best, while piling on the medication.

We Cushies later find that our constellation of symptoms are punctuated with abnormal test results hidden away in our patient charts. No one told us. Since our medical history stays with the office not the patient, one change in doctor leaves these clues to diagnosis behind.

This is why I suggest you take a list of symptoms to every appointment. You won't be boxed in by narrowly-focused questions based on one field of medicine, but you can instead partner with your doctors and discuss your symptoms list so you can get the care you deserve.

DON'T GET STUCK SEEING A SPECIALIST WHO TREATS ONLY ONE BODY SYSTEM. CUSHING'S AFFECTS THEM ALL.

DISCLAIMER: ME AND MY CORCEPT

I want to disclose my relationship with Corcept Therapeutics. I participated in their market research study in Philadelphia, CA in February 2012. I gave feedback on marketing material and told my story on camera for internal educational use. Corcept paid for my travel expenses. In July 2012, I met the Cushing's advocate and nurse at the MAGIC Convention in Chicago. We shared a meal as a group, and she kindly paid for our dinner. In addition, I hold stock in Corcept Therapeutics; not because I am rich, but because I have Cushing's.

Please rest assured that this affiliation will never compromise my intent to bring patients all information I can get my hands on to help them on their road to diagnosis and recovery. You have my word.